Overview
Pilomatrix carcinoma (also called pilomatrical carcinoma or malignant pilomatrixoma) is a very rare type of skin cancer that starts in the hair follicle. Specifically, it grows from cells that normally form the root sheath of hair. This cancer most often appears as a hard lump or mass under the skin, usually on the head, neck, or upper body. It can look similar to a benign (non-cancerous) growth called a pilomatrixoma, which makes it tricky to diagnose without a tissue sample. Pilomatrix carcinoma tends to grow slowly but can be locally aggressive, meaning it can invade nearby tissues. In some cases, it can spread to lymph nodes or other parts of the body, though this is uncommon. The tumor is usually firm, may feel calcified (hard like bone), and can sometimes ulcerate or break through the skin surface. Treatment is primarily surgical. The main goal is to remove the tumor completely with clear margins, meaning no cancer cells are left at the edges. Because this cancer is so rare, there are no standard chemotherapy or radiation protocols specifically approved for it, though these may be used in advanced cases. Early detection and complete surgical removal offer the best outcomes. Regular follow-up is important because the cancer can come back locally after surgery.
Also known as:
Key symptoms:
Hard lump or mass under the skinLump on the head, neck, face, or upper bodySkin that feels calcified or stony over the lumpSlow-growing bump that may suddenly grow fasterSkin ulceration or open sore over the lumpRedness or discoloration of the skin over the tumorLump that feels attached to deeper tissueSwollen lymph nodes near the tumor in advanced casesRecurrence of a lump after previous removal of a similar growth
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Pilomatrix carcinoma.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Pilomatrix carcinoma at this time.
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Specialists
View all specialists →No specialists are currently listed for Pilomatrix carcinoma.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Pilomatrix carcinoma.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.Was the tumor completely removed with clear margins, and what does that mean for my risk of it coming back?,How often do I need follow-up appointments, and what will you check for at each visit?,Should I have imaging tests like CT or MRI to check if the cancer has spread?,Is radiation therapy recommended in my case, and what are the benefits and risks?,Are there any genetic factors I should know about that could affect my family members?,What signs or symptoms should prompt me to contact you immediately between appointments?,Are there any clinical trials or research studies I might be eligible for?
Common questions about Pilomatrix carcinoma
What is Pilomatrix carcinoma?
Pilomatrix carcinoma (also called pilomatrical carcinoma or malignant pilomatrixoma) is a very rare type of skin cancer that starts in the hair follicle. Specifically, it grows from cells that normally form the root sheath of hair. This cancer most often appears as a hard lump or mass under the skin, usually on the head, neck, or upper body. It can look similar to a benign (non-cancerous) growth called a pilomatrixoma, which makes it tricky to diagnose without a tissue sample. Pilomatrix carcinoma tends to grow slowly but can be locally aggressive, meaning it can invade nearby tissues. In som
How is Pilomatrix carcinoma inherited?
Pilomatrix carcinoma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Pilomatrix carcinoma typically begin?
Typical onset of Pilomatrix carcinoma is adult. Age of onset can vary across affected individuals.